Meig's syndrome

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Diagnosis and reasoning

This post-menopausal lady has presented with ascites, a right sided pleural effusion, and a right sided pelvic mass - an ominous triad of features. The important diagnoses to be considered include Krukenberg's tumors secondary to breast or stomach carcinoma; ovarian carcinoma; Meig's syndrome; tuberculosis; and rarely, sarcoidosis. Her history is negative for symptoms of gastric carcinoma, while the unremarkable breast examination and normal mammogram exclude a breast malignancy. In addition, while ovarian cancer may metastasize to the lung, this is rather uncommon. Most often, even advanced ovarian carcinomas are localized to the peritoneal cavity. Meig's syndrome (where the ovarian tumor is benign) may give rise to this triad, and is a likely diagnosis. Disseminated tuberculosis with a tubo-ovarian mass and peritoneal involvement remains a rare possibility, as does sarcoidosis. Ultrasonography of the abdomen and pelvis is perhaps the best initial investigation - and in this patient shows the pelvic mass to be of right ovarian origin. While the presence of ascites and the echotexture of the mass favor a malignancy, the absence of intrabdominal lymphadenopathy is against this diagnosis (especially considering the size of the mass). Quantification of CA-125 (a tumor marker for ovarian malignancies) shows only a mild elevation. This is an equivocal result, as both benign and malignant ovarian tumors and even pelvic-peritoneal tuberculosis may give rise to CA-125 levels in this range. Pleural aspiration reveals protein and LDH (lactate dehydrogenase) levels which are markedly lower than those in the serum. This establishes the effusion to be a transudate. Note that malignancies and tuberculosis typically give rise to exudative effusions. The absence of malignant cells in the aspirate is further evidence against pulmonary metastases, while the negative quantiferon test and low ADA levels make tuberculosis unlikely. Note that endoscopy is not indicated, given the absence of gastrointestinal symptoms. Histopathology is required for a definitive diagnosis. Given the size of the mass and the possibility of malignancy, most clinicians would probably opt for a laparotomy. In addition, as the patient is postmenopausal, some clinicians might consider performing a total abdominal hysterectomy and bilateral salphingo-oophorectomy (TAH/BSO) instead of a right sided oophorectomy alone, as this may reduce the likelihood of future pelvic surgery. Note also that she will require drainage of the pleural effusion via an intercostal tube, prior to surgery under general anesthesia. Steroids and antituberculous therapy are not indicated here. The patient in this case eventually underwent a laparotomy + TAH/BSO. Subsequent histopathological analysis showed the ovarian tumor to be an ovarian fibroma (a form of benign tumor) - establishing the diagnosis to be Meig's Syndrome.


Discussion

Meig's Syndrome is defined as the triad of ascites, hydrothorax and a benign ovarian tumor. It is generally more common in elderly women, but may also occur in the young. The worldwide prevalence is unknown. Note that the ovarian tumor is always a benign solid tumor (most often an ovarian fibroma). These patients typically present with a non-productive cough, dyspnea, fatigue, bloating and weight loss. Premenopausal women may additionally complain of amenorrhea or menstrual irregularities. Examination typically shows ascites and a pleural effusion (which is right sided in the majority of patients). These are usually transudates, with cytology often showing reactive mesothelial cells. One school of thought postulates that irritation of the peritoneal surfaces by the ovarian tumor stimulates the secretion of peritoneal fluid, resulting in ascites. However, some authorities disagree with this, attributing the fluid secretion to direct pressure on the lymphatics or vessels; hormonal stimulation; or release of mediators by the tumor. The pleural effusion is thought to be secondary to passage of ascitic fluid into the pleural cavity through the diaphragm or diaphragmatic vessels. CA 125 levels in these patients may be normal or mildly elevated. Immunohistochemical studies suggest that these mild elevations are probably due to mesothelial expression of CA 125, rather than secretion by the ovarian tumor. Electrolyte abnormalities and a prolonged prothrombin time may also be present, while some patients may experience iron deficiency anemia. Meig's Syndrome is a benign condition amenable to permanent cure. The recommended management is surgical resection of the tumor, which subsequently results in spontaneous resolution of the ascites and pleural effusion within a few weeks. Life expectancy following management is similar to that of the general population.


Take home messages

  1. Meig's syndrome consists of the triad of a benign ovarian tumor, ascites and a pleural effusion.
  2. The clinical, biochemical and radiological findings associated with Meig's syndrome may mimic those of an ovarian malignancy.
  3. The effusions associated with the disease resolve in almost all patients once the tumor is surgically resected.
  4. Following treatment, the life expectancy is similar to that of the general population.

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  1. ACOG practice bulletin No 83: management of adnexal masses (2007)
  2. AFP: diagnosis and management of adnexal masses (2009)
  3. International journal of gynecological cancer: elevated CA125 level associated with Meig's syndrome: case report and review of the literature (2006)
  4. Journal of Oncology : Tumor Spreading to the Contralateral Ovary in Bilateral Ovarian Carcinoma Is a Late Event in Clonal Evolution (2010)
  5. RCOG green top guideline No 34: management of ovarian cysts (2003)
  6. Tuberculosis Diagnosis and Evaluation : Is CA-125 a reliable serum marker for diagnosis of tuberculosis ? (2005)